Department of Dermatology and Allergology, Philipps University Marburg, Germany.
Clin Immunol. 2010 Mar;134(3):320-30. doi: 10.1016/j.clim.2009.11.006. Epub 2009 Dec 16.
Pemphigus vulgaris (PV) is a severe autoimmune bullous skin disease and is primarily associated with IgG against desmoglein 3 (dsg3), a desmosomal adhesion protein. In light of the recent association of autoreactive T helper (Th) 2 cells with active PV, the present study sought to relate the occurrence of Th2-regulated dsg3-specific autoantibody subtypes, i.e. IgE and IgG4, in 93 well-characterized PV patients. Patients with acute onset PV (n=37) showed the highest concentrations of serum IgE and IgG4 autoantibodies, which were significantly lower in PV patients in remission (n=14). Furthermore, there was a strong correlation between dsg3-reactive IgE and IgG4 in acute onset, but not in chronic active (n=42) or remittent patients. Additionally, intercellular IgE deposits were detected in the epidermis of acute onset PV. Thus, dsg3-specific IgE and IgG4 autoantibodies are related to acute onset disease which provides additional support to the concept that PV is a Th2-driven autoimmune disorder.
寻常型天疱疮（PV）是一种严重的自身免疫性大疱性皮肤病，主要与针对桥粒芯糖蛋白 3（dsg3）的 IgG 抗体相关，dsg3 是一种桥粒黏附蛋白。鉴于最近报道的自身反应性辅助性 T 细胞（Th）2 细胞与活动性 PV 有关，本研究旨在研究 93 例明确诊断的 PV 患者中 Th2 调节的 dsg3 特异性自身抗体亚型（即 IgE 和 IgG4）的发生情况。急性发作的 PV 患者（n=37）血清 IgE 和 IgG4 自身抗体浓度最高，缓解期的 PV 患者（n=14）明显降低。此外，在急性发作患者中，dsg3 反应性 IgE 和 IgG4 之间存在强烈相关性，但在慢性活动性（n=42）或缓解性患者中则没有。此外，在急性发作的 PV 表皮中还检测到细胞间 IgE 沉积。因此，dsg3 特异性 IgE 和 IgG4 自身抗体与急性发作疾病相关，这为 PV 是一种 Th2 驱动的自身免疫性疾病的概念提供了额外的支持。
